Livedo reticularis

Livedo reticularis
Classification and external resources
ICD-10	R23.1
ICD-9-CM	782.61
DiseasesDB	7546
MedlinePlus	001478

Livedo reticularis is a common skin finding consisting of a mottled reticulated vascular pattern that appears as a lace-like purplish discoloration of the skin.^[1] The discoloration is caused by swelling of the venules owing to obstruction of capillaries by small blood clots. The blood clots in the small blood vessels can be a secondary effect of a condition that increases a person's risk of forming blood clots, including a wide array of pathological and nonpathological conditions . Examples include hyperlipidemia, microvascular hematological or anemia states, nutritional deficiencies, hyper- and autoimmune diseases, and drugs/toxins.

The condition may be normal or related to more severe underlying pathology.^[2] Its differential diagnosis is broadly divided into possible blood diseases, autoimmune (rheumatologic) diseases, cardiovascular diseases, cancers, and endocrine disorders. It can usually (in 80% of cases) be diagnosed by biopsy.^[3]

It may be aggravated by exposure to cold, and occurs most often in the lower extremities.

The condition's name derives from the Latin livere meaning bluish and reticular which refers to the net-like appearance.

Causes

A number of conditions may cause the appearance of livedo reticularis:

• Cutis marmorata telangiectatica congenita, a rare congenital condition
• Sneddon syndrome – association of livedoid vasculitis and systemic vascular disorders, such as strokes, due to underlying genetic cause^[4]
• Idiopathic livedo reticularis – the most common form of livedo reticularis, completely benign condition of unknown cause affecting mostly young women during the winter:^[5] It is a lacy purple appearance of skin in extremities due to sluggish venous blood flow. It may be mild, but ulceration may occur later in the summer.^[6]
• Secondary livedo reticularis:
  • Vasculitis autoimmune conditions:
    • Livedoid vasculitis – with painful ulceration occurring in the lower legs
    • Polyarteritis nodosa
    • Systemic lupus erythematosus^[7]
    • Dermatomyositis
    • Rheumatoid arthritis
    • Lymphoma
    • Pancreatitis^[8]
    • Chronic pancreatitis^[9]
    • Tuberculosis
  • Drug-related:
    • Adderall (side effect)
    • Amantadine (side effect)
    • Bromocriptine (side effect)
    • Beta IFN treatment, i.e. in multiple sclerosis^[10][11][12]
    • Livedo reticularis associated with rasagiline^[13]
    • Methylphenidate and dextroamphetamine-induced peripheral vasculopathy^[14]
    • Gefitinib^[15]
  • Obstruction of capillaries:
    • Cryoglobulinaemia – proteins in the blood that clump together in cold conditions^[16]
    • Antiphospholipid syndrome due to small blood clots
    • Hypercalcaemia (raised blood calcium levels which may be deposited in the capillaries)
    • Haematological disorders of polycythaemia rubra vera or thrombocytosis (excessive red cells or platelets)
    • Infections (syphilis, tuberculosis, Lyme disease)
    • Associated with acute renal failure due to cholesterol emboli status after cardiac catheterization
    • Arteriosclerosis (cholesterol emboli)^[17][18] and homocystinuria (due to Chromosome 21 autosomal recessive Cystathionine beta synthase deficiency)
    • Intra-arterial injection (especially in drug addicts)
  • Ehlers-Danlos syndrome – connective tissue disorder, often with many secondary conditions, may be present in all types
  • Pheochromocytoma^[19]
  • Livedoid vasculopathy and its association with factor V Leiden mutation^[20]
  • FILS syndrome (polymerase ε1 mutation in a human syndrome with facial dysmorphism, immunodeficiency, livedo, and short stature)^[21]
  • Primary hyperoxaluria, oxalosis (oxalate vasculopathy)^{[22][23][24][25][26]}
  • Cytomegalovirus infection (very rare clinical form, presenting with persistent fever and livedo reticularis on the extremities and cutaneous necrotizing vasculitis of the toes)^[27]
  • Generalized livedo reticularis induced by silicone implants for soft tissue augmentation^[28]
  • As a rare skin finding in children with Down syndrome^[29][30]
  • Idiopathic livedo reticularis with polyclonal IgM hypergammopathy^[31]
  • CO₂ angiography (rare, reported case)^[32]
  • A less common skin lesion of Churg-Strauss syndrome^[33]
  • Erythema nodosum-like cutaneous lesions of sarcoidosis showing livedoid changes in a patient with sarcoidosis and Sjögren's syndrome^[34]
  • Livedo vasculopathy associated with IgM antiphosphatidylserine-prothrombin complex antibody^[35]
  • Livedo vasculopathy associated with plasminogen activator inhibitor-1 promoter homozygosity and prothrombin G20210A heterozygosity^[35]
  • As a first sign of metastatic breast carcinoma (very rare)^[36]
  • Livedo reticularis associated with renal cell carcinoma (rare)^[37]
  • Buerger's disease (as an initial symptom)^[38]
  • As a rare manifestation of Graves hyperthyroidism^[39]
  • Associated with pernicious anaemia^[40]
  • Moyamoya disease (a rare, chronic cerebrovascular occlusive disease of unknown etiology, characterized by progressive stenosis of the arteries of the circle of Willis leading to an abnormal capillary network and resultant ischemic strokes or cerebral hemorrhages)^[41]
  • Associated with the use of a midline catheter^[42]
  • Familial primary cryofibrinogenemia.^[43]

Treatment

Other than identifying and treating any underlying conditions in secondary livedo,^[44] idiopathic livedo reticularis may improve with warming the area.

See also

• Livedoid dermatitis
• Perinatal gangrene of the buttock
• erythema ab igne (AKA livedo recticularis e calore) - a rash caused by prolonged heat exposure (e.g. hot water bottle or heat pad)

References

1. Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. p. 1615. ISBN 1-4160-2999-0.
2. "livedo reticularis" at Dorland's Medical Dictionary
3. Kroshinsky; Stone, John H.; Bloch, Donald B.; Sepehr, Alireza (February 2009). "Case 5-2009 — A 47-Year-Old Woman with a Rash and Numbness and Pain in the Legs". New England Journal of Medicine. 360 (7): 711–20. doi:10.1056/NEJMcpc0807822. PMID 19213685.
4. Sneddon, I. B. (April 1965). "Cerebro-Vascular Lesions And Livedo Reticularis". British Journal of Dermatology. 77 (4): 180–5. doi:10.1111/j.1365-2133.1965.tb14628.x. PMID 14278790. |first2= missing |last2= in Authors list (help)
5. Gibbs, Mark B.; English, Joseph C.; Zirwas, Matthew J. (2005). "Livedo reticularis: an update". J Am Acad Dermatol. 52 (6): 1009–19. doi:10.1016/j.jaad.2004.11.051. PMID 15928620.
6. Feldaker M, Hines E, Kierland R (1955). "Livedo reticularis with summer ulcerations". AMA Arch Derm. 72 (1): 31–42. doi:10.1001/archderm.1955.03730310033007. PMID 14387292.
7. Golden R (March 1963). "Livedo reticularis in systemic lupus erythematosus". Arch Dermatol. 87: 299–301. doi:10.1001/archderm.1963.01590150015002. PMID 13948706.
8. Sigmund W, Shelley W (1954). "Cutaneous manifestations of acute pancreatitis, with special reference to livedo reticularis". N Engl J Med. 251 (21): 851–3. doi:10.1056/NEJM195411182512104. PMID 13214346.
9. Gould, Jennifer W.; Helms, Stephen E.; Schulz, Susan M.; Stevens, Seth R. (1998). "Relapsing livedo reticularis in the setting of chronic pancreatitis". Journal of the American Academy of Dermatology. 39 (6): 1035–1036. doi:10.1016/S0190-9622(98)70290-7. PMID 9843029.
10. Rot, Uroš; Ledinek, Alenka Horvat (December 2013). "Interferons beta have vasoconstrictive and procoagulant effects: a woman who developed livedo reticularis and Raynaud phenomenon in association with interferon beta treatment for multiple sclerosis". Clinical Neurology and Neurosurgery. 115 (Suppl 1): S79–81. doi:10.1016/j.clineuro.2013.09.027. PMID 24321162.
11. Fox, Michelle; Tahan, Steven; Kim, Caroline C. (2012). "Livedo Reticularis: A Side Effect of Interferon Therapy in a Pediatric Patient with Melanoma". Pediatric Dermatology. 29 (3): 333–5. doi:10.1111/j.1525-1470.2011.01426.x. PMID 21575046.
12. Cruz, Boris Afonso; de Queiroz, Eustáquio; Nunes, Simone Vilela; Cruz Filho, Achiles; Campos, Gilberto Belisario; de Carvalho Monteiro, Ernesto Lentz; Crivellari, Humberto (2000). "Fênomeno de Raynaud grave associado a terapia com interferon-beta para esclerose múltipla: relato de caso" [Severe Raynaud's phenomenon associated with interferon-beta therapy for multiple sclerosis: case report]. Arquivos de Neuro-Psiquiatria (in Portuguese). 58 (2B): 556–9. doi:10.1590/S0004-282X2000000300025. PMID 10920422.
13. Strowd, Lindsay C.; Lee, Andrew D.; Yosipovitch, Gil (June 2012). "Livedo Reticularis Associated With Rasagiline (Azilect)". Journal of Drugs in Dermatology. 11 (6): 764–5. PMID 22648227.
14. Syed, Reema H.; Moore, Terry L. (2008). "Methylphenidate and Dextroamphetamine-Induced Peripheral Vasculopathy". JCR: Journal of Clinical Rheumatology. 14: 30–33. doi:10.1097/RHU.0b013e3181639aaa.
15. Blume, Jonathan E.; Miller, Craig C. (2007). "Livedo reticularis with retiform purpura associated with gefitinib (Iressa®)". International Journal of Dermatology. 46 (12): 1307–8. doi:10.1111/j.1365-4632.2007.03319.x. PMID 18173531.
16. Langhof H, Braun G, Matzkowski H (1957). "Livedo reticularis durch Kältegelierung des Blutes bei γ-Plasmocytom" [Livedo reticularis due to cold gelation of the blood by gamma-plasmacytoma]. Archiv für klinische und experimentelle Dermatologie (in German). 205 (4): 343–50. doi:10.1007/BF00693523. PMID 13522017.
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18. Stewart W, Lauret P, Testart J, Thomine E, Boulliê M, Leroy D (1977). "Les manifestations cutanées des emoblies de critaux de cholestérol" [Cutaneous cholesterol emboli]. Ann Dermatol Venereol (in French). 104 (1): 5–8. PMID 843026.
19. Buckley, Sarah A.; Lessing, Juan N.; Mark, Nicholas M. (2013). "Livedo Reticularis in a Patient with Pheochromocytoma Resolving After Adrenalectomy". The Journal of Clinical Endocrinology & Metabolism. 98 (2): 439–40. doi:10.1210/jc.2012-2842.
20. Yong AA, Tan AW, Giam YC, Tang MB (December 2012). "Livedoid vasculopathy and its association with factor V Leiden mutation" (PDF). Singapore Med J. 53 (12): e258–60. PMID 23268168.
21. Pachlopnik Schmid, Jana; Lemoine; Nehme, Nadine; Cormier-Daire, Valéry; Revy, Patrick; Debeurme, Franck; Debré, Marianne; Nitschke, Patrick; Bole-Feysot, Christine; Legeai-Mallet, Laurence; Lim, Annick; de Villartay, Jean-Pierre; Picard, Capucine; Durandy, Anne; Fischer, Alain; de Saint Basile, Geneviève (2012). "Polymerase ε1 mutation in a human syndrome with facial dysmorphism, immunodeficiency, livedo, and short stature ('FILS syndrome')". Journal of Experimental Medicine. 209 (13): 2323–30. doi:10.1084/jem.20121303. PMC 3526359. PMID 23230001.
22. Jorquera-Barquero, E.; Súarez-Marrero, M.C.; Fernández Girón, F.; Borrero Martín, J.J. (2013). "Oxalosis y livedo reticularis" [Oxalosis and Livedo Reticularis]. Actas Dermo-Sifiliográficas (in Spanish). 104 (9): 815–8. doi:10.1016/j.ad.2012.04.019. PMID 23103120.
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24. Marconi V, Mofid MZ, McCall C, Eckman I, Nousari HC (February 2002). "Primary hyperoxaluria: report of a patient with livedo reticularis and digital infarcts". J. Am. Acad. Dermatol. 46 (2 Suppl Case Reports): S16–8. doi:10.1067/mjd.2002.105475. PMID 11807460.
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26. Singh S, Tai C, Ganz G, et al. (April 1999). "Steroid-responsive pleuropericarditis and livedo reticularis in an unusual case of adult-onset primary hyperoxaluria". Am. J. Kidney Dis. 33 (4): e5. doi:10.1016/s0272-6386(99)70246-5. PMID 10196036.
27. Arslan, Ferhat; Batirel, Ayse; Mert, Ali; Ozer, Serdar (2012). "Cytomegalovirus (CMV)-related cutaneous necrotizing vasculitis: Case report and literature review". The Brazilian Journal of Infectious Diseases. 16 (5): 482–5. doi:10.1016/j.bjid.2012.08.002. PMID 22975173.
28. Camacho, Diana; Machan, Shalma; Pilesanski, Ursula; Revelles, Juan Maria; Martín, Lucia; Requena, Luis (2012). "Generalized Livedo Reticularis Induced by Silicone Implants for Soft Tissue Augmentation". The American Journal of Dermatopathology. 34 (2): 203–7. doi:10.1097/DAD.0b013e31821cb3c5. PMID 22441370.
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30. Daneshpazhooh M, Nazemi TM, Bigdeloo L, Yoosefi M (2007). "Mucocutaneous findings in 100 children with Down syndrome". Pediatr Dermatol. 24 (3): 317–20. doi:10.1111/j.1525-1470.2007.00412.x. PMID 17542890.
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36. Gambichler, T.; Baier, P.; Altmeyer, P. (2009). "Generalized livedo reticularis as the first sign of metastatic breast carcinoma". Clinical and Experimental Dermatology. 34 (2): 253–4. doi:10.1111/j.1365-2230.2008.02801.x. PMID 19120398.
37. Erel, Arzu; Ozsoy, Esra; University, Gazi (2001). "Livedo reticularis associated with renal cell carcinoma". International Journal of Dermatology. 40 (4): 299–300. doi:10.1046/j.1365-4362.2001.00895.x. PMID 11454094.
38. Takanashi, Tetsuo; Horigome, Reiko; Okuda, Yasuaki; Nose, Masato; Matsuda, Masayuki; Ikeda, Shu-Ichi (2007). "Buerger's Disease Manifesting Nodular Erythema with Livedo Reticularis". Internal Medicine. 46 (21): 1815–9. doi:10.2169/internalmedicine.46.0143. PMID 17978541.
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External links

• DermNet